Munch's Scream Sign: An Unusual Case of Dysphagia in Advanced Dementia.

Abstract

To the Editor: An 83-year-old woman admitted because of gradual, nonresponsive decline in her general condition over recent weeks presented with dysphagia and weight loss. She was referred from a nursing home, where she had been resident for 5 years. She had advanced dementia (Clinical Dementia Rating 4) due to Alzheimer's disease, and over the previous 2 weeks, she had developed loss of appetite and dysphagia. Parenteral feeding was started through central venous access. On admission, a multidimensional assessment showed total lack of autonomy in daily functioning (activities of daily living 0/6, Barthel Index 8/10), severe cognitive impairment (Mini-Mental State Examination not administrable) with behavioral disturbances (Neuropsychiatric Inventory (NPI) 54/144) and poor nutritional status (Mini Nutritional Assessment 13/30, body mass index 17.8 kg/m2, 3-kg weight loss recorded in last month). According to Pain Assessment in Advanced Dementia (PAINAD) she had severe pain (PAINAD 8/10). Upon physical examination on admission, she presented with senile cachexia, expressive aphasia, and muscular tone spasticity. Forced position of her mouth was found, with inability to fully occlude the jaw, concomitant mandibular deviation to the right on full aperture, and hypomobility of the right temporomandibular joint (TMJ) (Figure 1). Compression of the right TMJ produced pain, which revealed a dislocation of the right TMJ. She was referred to a maxillofacial surgeon, who manually reduced the joint without anesthesia. After the procedure and 10 days of jaw immobilization, she started to eat without any swallowing disorders and recovered her ability to communicate, although still impaired by the underlying advanced dementia. Her behavioral disturbances mostly disappeared (NPI 18/144), and pain was no longer relevant (PAINAD 2/10). The clinical symptoms of TMJ dislocation include changes to occlusion and reduction of mastication function. Once the dislocation of the TMJ occurs bilaterally or unilaterally, the mouth cannot be closed without pain, the tension of temporalis and masseter increases dramatically, and pronunciation is impossible. This gives rise to a typical facial expression, curiously similar to a well-known painting by Munch, thereby inspiring the unconventional, semeiologically derived term “Munch's scream sign.”1, 2 Lateral dislocation of the joint may be treated simply through manual reduction, but sometimes it develops into a chronic condition and treatment is difficult because spasming of the masticatory muscles exacerbates the presentation and increases the thickness of the tissue posterior to the joint disc. Surgical treatment of TMJ dislocation may be considered if the dislocation occurs recurrently or when nonsurgical treatment fails.3 This case report is relevant because it occurred in an elderly woman with advanced dementia, whose communicative and functional abilities were severely lacking. When considering care in dementia, clinicians are frequently faced with situations that question the very nature of suffering. The inability of individuals to convey their feelings accurately complicates efforts to identify and measure the nature of their discomfort. The diminished capacity of individuals with dementia to advocate for themselves increases the duty of care of those charged with this responsibility. This report highlights some major concerns in caring for dementia: When physical conditions worsen and differ from the previous state, clinicians should consider a wide spectrum of differential diagnosis. If a person with dementia develops distressing noncognitive symptoms of dementia, he or she should be assessed to identify possible contributing factors, triggers or unmet needs.4 In individuals with advanced dementia, if pain, assessed through observational scales or clinical examination, is suspected, analgesic treatment should always be considered.5 Therefore pain should always be assessed in noncommunicative individuals.6 There is a high risk of misleading diagnoses and undertreatment in dementia, especially in noncommunicative individuals. In particular, changes in behavior and physical function in elderly adults with dementia must always be considered as a sign of a problem. It is essential that continuous education and training programs be developed, implemented, and evaluated to ensure the best practice in caring people with dementia. Moreover, in a world in which people are frequently spectators to the demise of physical examination, this report represents a significant example of a cost-effective and simply obtained diagnosis without the need for costly and time-consuming diagnostic tests. Conflict of Interest: The authors have no financial or any other kind of personal conflicts with this paper. Written consent for the image and case report was obtained from the woman's daughter. Author Contributions: All authors contributed to the preparation of this manuscript. Sponsor's Role: None.